Product Name :
Dyskerin (K203) polyclonal antibody Background :
Dyskerin (NAP57) associates with the chaperone protein Nopp140 and forms a small ribonucleoprotein particle with GAR1 (NOLA1), NHP2 (NOLA2) and Nop10 for the isomerization of uridine to pseudouridine. GAR1, NHP2 and Dyskerin localize to the dense fibrillar component of the nucleolus and in nuclear Cajal bodies. The Dyskerin gene maps to chromosome Xq28. Missense mutations in the Dyskerin gene interfere with normal nuclear localization of Dyskerin and cause Dyskeratosis congenita (DKC). DKC is a rare, X-linked bone marrow disorder characterized by cutaneous hyperpigmentation, dystrophy of the nails, atrophy of the testicles and leukoplakia of the oral mucosa. The GAR1 gene maps to chromosome 4q25 and encodes a 28 kDa protein. The NHP2 gene maps to chromosome 5q35.3 and encodes a 155 amino acid protein Product :
Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2 Storage&Stability :
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles. Specificity :
Dyskerin (K203) polyclonal antibody detects endogenous levels of Dyskerin protein. Immunogen :
Synthetic peptide, corresponding to amino acids 171-220 of Human Dyskerin. Conjugate :
Unconjugated Modification :
Unmodification
Dyskerin (K203) polyclonal antibody Background :
Dyskerin (NAP57) associates with the chaperone protein Nopp140 and forms a small ribonucleoprotein particle with GAR1 (NOLA1), NHP2 (NOLA2) and Nop10 for the isomerization of uridine to pseudouridine. GAR1, NHP2 and Dyskerin localize to the dense fibrillar component of the nucleolus and in nuclear Cajal bodies. The Dyskerin gene maps to chromosome Xq28. Missense mutations in the Dyskerin gene interfere with normal nuclear localization of Dyskerin and cause Dyskeratosis congenita (DKC). DKC is a rare, X-linked bone marrow disorder characterized by cutaneous hyperpigmentation, dystrophy of the nails, atrophy of the testicles and leukoplakia of the oral mucosa. The GAR1 gene maps to chromosome 4q25 and encodes a 28 kDa protein. The NHP2 gene maps to chromosome 5q35.3 and encodes a 155 amino acid protein Product :
Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2 Storage&Stability :
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles. Specificity :
Dyskerin (K203) polyclonal antibody detects endogenous levels of Dyskerin protein. Immunogen :
Synthetic peptide, corresponding to amino acids 171-220 of Human Dyskerin. Conjugate :
Unconjugated Modification :
Unmodification
Bioworld Biotech only provide peptides for our antibodies and do not provide additional peptide customization services.
Price/Size :
USD 368/1mg/vial
Tips:
For phospho antibody, we provide phospho peptide(0.5mg) and non-phospho peptide(0.5mg).Describe :
Blocking peptides are peptides that bind specifically to the target antibody and block antibody binding. These peptide usually contains the epitope recognized by the antibody. Antibodies bound to the blocking peptide no longer bind to the epitope on the target protein. This mechanism is useful when non-specific binding is an issue, for example, in Western blotting (WB) and Immunohistochemistry (IHC). By comparing the staining from the blocked antibody versus the antibody alone, one can see which staining is specific; Specific binding will be absent from the western blot or IHC performed with the neutralized antibody.Formula:
Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 10 mg/ml.The purity is >90%,tested by HPLC and MS.
Storage:
The freeze-dried powder is more stable. For short time at 2-8°C. For long term storage store at -20°C.
Note :
This product is for research use only (RUO only). Not for use in diagnostic or therapeutic procedures.